ABSTRACT

Morquio syndrome is a subtype of mucopolysaccharidoses that is characterised by intracellular keratine sulphate accumulation and is inherited in an autosomal recessive manner. This accumulation causes anatomical distortion in the upper airway and therefore results in difficult or impossible endotracheal intubation. In this article, pre-anaesthetic preparations and general anaesthesia management of a patient with Morquio syndrome for whom decompression of craniocervical junction and surgical correction of C1-2 subluxation because of odontoid hypoplasia was planned is reported.

Keywords: Morquio Syndrome, anaesthesia, difficult airway